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RHEUM&VASCULITIS. > VASCULITIS > Flashcards

VASCULITIS Flashcards

0
Q

what are the examples of immune mechanisms in vasculitic syndromes?

A
  1. deposition of immune complexes
  2. direct attack on vessels by circulating ABs
  3. Various forms of cell mediated immunity
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1
Q

WHAT IS VASCULITIS?

A

Inflammation and necrosis of blood vessels by: 1. Immune mechanisms

  1. . infectious agents
  2. mech trauma
  3. radiation-toxins
    * Viral infections sometimes
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2
Q

human vasculitis is associated with which viruses?

A
  1. HBV
  2. Parvovirus
  3. CMV
  4. Herpes Simplex virus
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3
Q

Small vessel Vasculitidies are assoc. with …?

ANCA- Go full board to get rid of the infl response

except(all Immune complex vasculitis):
Cyroglobulinemia Vasculi
IgA Vasc (Henoh-Schonlein) : usually self limiting, so conservative therapy.
Hypocomplementemic Urticarial Vasculitis (Anti-C1q Vasculitis)

A

Antineutrophil cytoplasmic Antibodies;” ANCA”

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4
Q

Does Hep B cause c-ANCA or p-ANCA?

A

HepB causes Atypical P-ANCA.

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5
Q

What is cryoglobulinemia and what are the types?

Protein that precipitates in small blood vessels at higher concentration and stasis.. also at cold temperatures when this pt’s blood is drawn.
- radian hand, drop foot, may be present.

A

Cryoglobulin: +pupura that doesnt blanch in the cold, may also involve the kidney-> necrotizing glomerulonephritis!

Type 1: monoclonal eg in chronic

Type 2: Mixed cryoglobinemia.
Monoclonal IgM Rh factor binds polyclonal IgG. eg in ~80% Hepatitis C. RH factor in HCV cryoglobulinemia shows low affinity maturation. BASICALLY- A FAILURE OR THE T CELL HELP therefore no maturation and no IgG formation! –> High assoc. w/ Lymphoma!!

Type 3: Oligo and Polyclonal . eg. in Pts with lupus or other autoimmune conditions.

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6
Q

ANCA Pathology involves which cells?

A

T cell!

ANCA Assoc. vasculitis often in small vessels.

Wegeners: upper resp. symptoms: sinuses, Lower resp. symptoms: Lung disease, Glomerular nephritis (kidney disease)

Microscopic Polyangiitis: + lung disease

Churg- Strauss: granuloma(fleeting lung infiltrates)+ eosinophils+ Asthma. (reaction to unknown antigen)

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7
Q

“socks and gloves”

A

Affects the hands and feet, in the same pattern that the gloves and socks would cover.

involve peripheral neuropathy eg from diabetes , ETOH(direct effect on the nerves, can cause mononeuritis multiplex), and Hep B,C , HIV, Lupus and other autoinflammatory conditions.

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8
Q

how do we rule out a stroke, with symmetric symptoms eg hand and foot drop on the same side.

A
  1. Muscle wasting!!

Lower motor neuron wld have fasciculation and the pt is stiff, with preserved and increased reflexes -> the nerve is dead!!

Stroke –> upper motor neuron,no fasciculations, . LISTEN TO LECTURE!!

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9
Q

APE HAND!!

A

MEDIAN NERVE!!

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10
Q

RADIAL HAND!! - DAAH!

A

RADIAL NERVE. Cld be from lime disease! esp if the pt is from an endermic area!

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11
Q

Foot drop

A

perineal nerve.

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12
Q

KAWASAKI!!

A

Strawberry tongue in kids!

size of vessels affected- medium sized vessels, affects mostly kids under 5.

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13
Q

look up the scaling of muscle function.

A 
0 no movt
1 flicker
2 horizontal, no gravity
3 w/ gravity, no resistance
4 w/ gravity + resistance
5 Normal
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14
Q

fever, anemia, fatigue, headache + jaw claudication.. “PAIN CHEWING”

in Pt > 50 yrs old.

Note: these first 4 symptoms symptoms cld be rom ENDOCARDITIS! or a similar infectious process.

A

Temporal artery biopsy! Cld be GCA.

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15
Q

TAKAYASU Arteritis.
pts hyper developed in upper extremities
Born with aorta coactation
poor upper pulses, good lower pulses.

A

large arteries pathology in pts <50yrs
claudication in upper extremities
low grade fever, anemia, fatigue

other possiblities: infection, malignancy

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16
Q

TREATMENT of vasculitis.

1st- you have to know which one you are dealing with, even though the therapy is usually the same.

ALWAYS HAVE TO HAVE A URINE ANALYSIS! INDICATION OF VASCULITIS IN KIDNEY! SO watch the CK, esp in polyangitiis Nodosa –> which wld cause protenuria and hematuria!!

A

Immunosuppresion therapy immediately if the infectious agent is a virus!
Previously gave cyclophosphomide, except GCA which need steroids.
Kawasaki –> Aspirin

T cell induced cases are treated with B cell killers, eg rituximab.

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17
Q

when to plasma exchange?

A

Not in acute infection cases, but good to clear the immune complexes.

This is also considered immunocompromising.

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18
Q

info.

A

bowel, skin, kidney, nerve :medium, mononeuropathy.

sensory : small vessels

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19
Q

heavy smoker vascular pathology?

DONT GIVE STEROIDS!

A

prone to Thromboangitiis Obliterans “Buerger’s disease”
Its is large vessel pathology, not vasculitis.

These pts dont have systemic symptoms, vessel walls are fine, the inflammatory cells are in the thrombi formed in the vessels!!

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20
Q

how wld you tell a current Hep infection from a vaccinised persone, from a carrier?

A

Antibody against core antigen –> shows the end of an infection or you have a secondary / reinfection.

+Antigen : carrier or current infection.

+ve Antibody and core antigen +ve: current infection.

21
Q

CLINICAL FEATURES OF VASCULITIS (pthma)

A
  1. Non specific inflammation symptoms, (fever, fatigue,Wt. loss,
  2. Symptoms of organ ischemia from luminal narowing or thrombosis of the inflammed vessels.
22
Q

types of vasculitis

A

Large vessel : Aorta& its major branches:
T cell mediated, type 4 hyper reactions!

Medium sized vessels : Muscular arteries that supply organs
small vessel : arterioles, capillaries & venules

23
Q

Large vessel vasculitis. (All w/ granulomatous inflammation respond well to corticosteroids!!)

1.Temporal (GIANT CELL)Vasculitis
culprit: T cell inflitrates!
RISK: BLINDNESS
TX: CORTICOSTEROIDS

+giant cells in intima,media is fibrotic in late stage.

A
  1. Most common in pple >50yrs, esp women! age onset is 70 yrs.
  2. ANCA is absent
  3. classically: Affects the carotid artery branches.

pathogenesis: granulomatous inflamm starting in adventitia, and then myofibroblast prolif –> lumen constriction.

  1. presents as:
    headache (temporal artery), Visual disturbances (opthl. artery), jaw claudication, flu like smptoms w/ joint(shoulders an hips) and muscle pain(polymyalgia rheumatica)
    ESR ELEVATED
24
Q

Large vessel vasculitis.
2. Takayasu Arteritis “Pulseless disease” of the aorta and its branches.
Risk: CHF in severe cases, BLINDNESS;loss of visual acuity, clue: asymmetric BP or loss of pulses
Tx:Corticosteroids for early intervention. Late intervention requires surgery.

A

1.Granulomatous Vasculitis that classically affects the Aortic Arch at branch points.

  1. Present in mostly young asians WOMEN(90%) severe cardiac symptoms+ intermittent claudication of the arms and legs.
    * *ESR ELEVATED**
25
Q

Medium size Vessel Vasculitis
(and some small arteries,with occasional large vessels affected too)
1.Polyarteritis Nodosa :NOT DIAGNOSES W/ A BIOPSY! DO AN ANGIOGRAM TO SEE THE ANEURYSM & INFARCT.
note: lungs are spared
Risk: FATAL!!
TX: Corticosteroids, cyclophosphomide

A

Necrotizing vasculitis involving multiple organs: kidney, heart, muscle, skin, mesentery… MORE COMMON IN MEN.

Classically: presents in young adults as hypertension(renal artery involvement), abdominal pain w/ melena (mesentaric artery involvement), Neurologic disturbances, and skin lesions –>Like the vessels, Skin heals w/ fibrosis –> forming a “string of pearls”
Assoc. w/Serum HBsAg

26
Q

Medium Sized Vasculitis

  1. KAWASAKI Disease (.. ACUTE NECROTISING VASCULITIS of infancy and early childhood!)
    Risk: coronary artery involvement is common & leads to:
    a)Thrombosis w/ MI
    b)Coronary artery aneurysm w/ rupture
    TX: Aspirin and IVIG; disease is self limiting.
A

Classically affects: Asian Children

27
Q

Medium Sized Vasculitis
3. Buerger Disease “ Thromboangitiis Obliterans” - Peripheral vascular disease of smokers.

Risk:Autoamputation of fingers and toes, Raynaud Phenomenon
TX: Smoking cessation!

A

Necrotizing Vasculitis involving digits ; distal arm and legs.

Pathogenesis:

presentation:
start btwn 25-40 as intermittent claudication aft exercise, which is relieved by resting.
Painful Ulceration on digit –> gangrene, autoamputation of fingers and toes, Raynaud’s phenomenon.

28
Q

All Medium Vessel Vasculitis

A

Polyarteritis Nodosa
Kawasaki
Buerger’s

29
Q

All large vessel Arteritis (2)

A

Takayasu Syndrome

Temporal /Giant cell arteritis

30
Q

All small vessel Vasculites (all ANCA +ve)

A

Microscopic Polyangiitis
Wegener Granulomatosis
Churg-Strauss Syndrome.

31
Q

Small vessel vasculities

  1. Microscopic Polyangiitis
    Risk:
    Tx:cortiosteroids and cyclophosphomide; relapse is common
A
  1. Necrotising vasculitis that affects many organs, especially the lungs and kidneys!
  2. Serum p-ANCA corr. w/ disease activity.

Classic presentation:
Middle aged male w/hemoptysis w/ bilateral nodular lung infiltrates, hematuria due to a rapidly progressing glomerulonephritis.

32
Q

Small vessel vasculitis

  1. Churg-Straus Syndrome. “ systemic vasculitis of young pts w/ asthma”
    Risk: Poor prognosis if untreated.
    TX: Corticosteroids
A
  1. necrotizing granulomatous inflammation w/ intense eosinophilic infiltrates in and around blood vessels
    1a. Involves multiple organs, esp the lungs and heart.
  2. p-ANCA levels corr. w/ disease activity
  3. presentation:
    Asthma, Peripheral eosinophilia
33
Q

Small vessel vasculitis

3.Wegener’s Granulomatosis
- Saddle nose!
“ vasculitis of the resp. tract & kidney”
Risk: DEATH W/ 1 YR! mean survival of 5-6moths
Tx:Cyclophospomide achieves complete remission and substantial disease free intervals in most pts.

A

1.Necrotizing granulomatous vasculitis involving the nose,sinuses, nasopharynx, lungs and kidneys(renal glomerular disease)
More men than women affected ~ 50-60y.o
2. Serum c-ANCA levels corr w/ the disease activity
Biopsy –> Large necrotizing granuloma w/ adjacent necrotizing vasculitis.

3.classic presentation:
Middle aged ale w/ Sinusitis or nasopharyngeal ulceration, hemoptysis w/ bilateral nodular lung infiltrates wc mimicks TB**, hematuria from rapidly progressing glomerulonephritis

34
Q

Small vessel vasculitis

  1. Henoch-Schonlein Purpura
    Risk:
    Tx: Steroids if severe; usually self limiting.
A

Most common vasculitis in kids!!
Caused by IgA Immune complex deposition

presents with:
Palpable purpura on buttocks and legs.
GI pain & bleeding, Hematuria(IgA nephropathy) which usually occurs after an upper resp tract infection.

35
Q

(HTN)

Press >140/90 mm Hg

A

primary:95% due to unknown etiology

Secondary: 5% due to identifiable cases eg. renal artery stenosis(engages the renin, Ang 2, Aldosterone effects to incr. BP)

Secondary HTN classically has high plasma renin and unilateral atrophy due to low blood flow of the affected kidney.

Stenosis causes: Atherosclerosis(elderly men) or Fibromuscular dysplasia (young females)

Fibromusc dysplasia: developmentaldefect–> ireeg thickening of large n med sized vessels, esp the renal artery

36
Q

Arteriosclerosis “Hard arteries”

what are the 3 pathologic patterns?

A
  1. Atherosclerosis (intima plaque obtrsucting blood flow)
  2. ArterioLOsclerosis (Narrowing of small arterioles )
  3. Monckeberg Medial calcific sclerosis(Non obstructive calcification of the media of muscular (medium sized) arteries)
37
Q

Arteriosclerosis

  1. Atherosclerosis
    Pathogenesis..
A

Intimal plaque consists of a necrotizing lipid core (mostly cholesterol) w a fibromuscular cap.

pathogenesis:

  1. damage to the endothelium allows lipids to leak into the intima
  2. oxidized lipids are then consumed by macrophages –> resulting in form cells
  3. inflamm & healing –> deposition of ECM and prolif of smooth muscle.
38
Q

Arteriosclerosis

1b. Atherosclerosis
Morphology:

A

Begins as fatty streaks(yellow lesions of the intima; lipid ladden macrophages) –>progresses to plaque.

39
Q

arteriosclerosis

  1. Arteriolosclerosis

types and pathogenesis…

A

narrowing of small vessels –> 2 types.

  1. hyaline types: proteins leaking into the vessel walls(appear pink on microscopy) –> thickening –> reduced vessel caliber –>end organ ischemia, classically: glomerular scarring–>chronic real failure.
  2. hyperplastic types: Thickening from hyperplasia of smooth muscles “onionskin appearance”–> end organ ischemia,classically: Acute renal failure w/ xteristic “flea bitten” appearance.
40
Q

Arteriosclerosis.

  1. Monckerberg Medial Calcification
A

1.calcification of the media of muscular (med. sized) arteries.

  1. Nonobstructive
  2. Not clinically significant.
41
Q

what is hypersensitivity angiitis?

A

Broad range of iflamm, vascular lesions thought to result from a reaction to aforeign object.

Presentation:
cutaneous vasculitis(reaction from penicillin, aspirin, thiazide...): palpable purpura, typically on the lower extremities.
42
Q

Systematic vasculitis

Behcets disease
Vasculitis mainly involving the major mucous membranes of many organs.

targets both large n small vessels.
Tx:corticosteroids.

A

Systemic vasculitis

xterized by:
Oral aphthous ulcers, Genital ulcers, ocular inflammation, CNS lesions, GI and CVS system

43
Q

Radiation vasculitis

risk: predisposes to accelerated atherosclerosis.

A

Acute phase:
endothelial injury and denudation, ballooning degen of the intimal smooth muscle cells, and medial smooth muscle necrosis, wc may be fibrinoid.

Chronic phase: intimal hyperplasia, fibrosis of the vessel wall w/ complete fibrous occlusion sometimes.

44
Q

Rickettsial vasculitis

Cause: intracellular Parasites

A

organisms usually disseminate from the entry site into the blood and invade the endothelial cells, smooth muscle cells of the media of SMALL VESSELS AND CAPILLARIES.

45
Q

Good Pasture Syndrome.

Pulmonary Hemorrhage and glomeruonephritis.

A

Auto ABs against Basement membranes in some tissues.

Pulmonary- renal syndrome

46
Q

notes on Purpura

A

Cld be meningitis in Kids…
HSP (P for purpura) –> IgA deposition.

DDX-
Non infectious purpura: + necrotizing
Allergic reaction? i.e hypersensitivity vasculitis

47
Q

Hematuria

A

Necrotizing Glomeruli

48
Q

NADPH def.

A

Chronic Granulomatous inflammation

Neutrophils cant kill! so bring on the T cells!

49
Q

more notes on Churg-Strauss….

A

*** can be +ve p-ANCA(small vessel vasculitis) or -ve ANCA **

ADULT ONSET STEROID ASTHMA (IN THE 40s) –> Churg -Strauss.
Eosinophils melt away with Steroids.

note: In leukotriene Inhibitor taking Asthma pts, as opposed to steroids –> Churg Strauss results!

RHEUM&VASCULITIS. (3 decks)

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